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Ruth-Mary deSouza trained in medicine at Closed Spinal Dysraphism Guy’s, Kings and St Thomas Medical School and graduated in 2008. She entered the London and Tethered Cord Neurosurgery training programme in 2010 and is currently an ST5 trainee on the South Thames Syndrome: A Review of Neurosurgery programme. David Frim Multidisciplinary Team Management is Professor of , Neurology and Paediatrics at the University of Chicago. He is an Summary internationally recognised • Embryology of spinal dysraphism clinical Neurosurgeon and Neurosciences Researcher • Clinical features of tethered cord syndrome who specialises in the care • Multidisciplinary management of closed spinal dysraphism of children and adults with congenital neurosurgical problems. Currently, Dr Frim serves as principal investigator on laboratory studies related to neural injury and clinical studies focusing on Abstract outcomes after treatment of congenital anomalies of the especially as related to cognition. The initial diagnosis as well as the long term management of occult spinal dysraphism and Dr Frim is joint senior author of the article. tethered is often managed by a large number of healthcare professionals including Paediatricians, GPs, Neurologists, Neurosurgeons, Rehabilitation Physicians and Paige Terrien Church Therapists. We review the entity of spinal dysraphism. An approach to the evaluation and is an Assistant Professor of diagnosis of these entities is subsequently discussed. In addition, concepts involved in the Paediatrics at the pathophysiology, neurosurgical repair, and outcome are presented in the context of postop - University of Toronto. She is the Director of the erative management issues that rely upon the knowledge of all professionals who may Neonatal Follow Up Clinic encounter these patients. at Sunnybrook Health Sciences Centre and the Introduction Developmental Behavioral Physician Lead in the Spina The finding of a midline spinal anomaly in a child typically prompts referral to the Paediatric Bifida clinic at Holland Neurosurgeon for the evaluation of an occult spinal dysraphic state. Unfortunately, occult Bloorview Kids spinal dysraphism is not always readily apparent on physical examination, but is often diag - Rehabilitation Hospital. Dr Church is board certified nosed retrospectively after the child presents with neurologic, urologic, and orthopaedic find - through the American Board of Paediatrics in Neonatology, and Developmental Behavioral ings. In this article, we review the pathology and pathophysiology of occult spinal dysraphism Paediatrics and is interested in long-term functional and its relationship to the clinical entity of the tethered cord syndrome. Subsequently, outcomes of infants with neurologic conditions. concepts in the surgical and biopsychosocial management of children born with these defects will be discussed, in the context of management by the multidisciplinary team Tony Elias including Neurologists, GPs, Paediatric Surgeons, Physiotherapists and Neurorehabilitation MCh, FRCS has been Specialists. trained in Neurosurgery in India, and has obtained Fellowship in Definitions Paediatric Neurosurgery Spinal dysraphism is an umbrella term that describes any anomaly of the spinal cord, cauda from Great Ormond Street equina or overlying tissues such as vertebrae, muscles and skin. The nervous system abnor - Hospital, London and mality may or may not have associated mesenchymal or dermal changes. 1,2,3 Spinal Spinal Fellowship from National Hospital for dysraphism is essentially an anatomical term describing a spectrum of lesions and associ - Neurology and ated pathology – tethered cord is the clinical manifestation of the anatomical abnormalities Neurosurgery, London. He that constitute spinal dysraphism. Spinal dysraphism, also called , can be subdi - is a Consultant Paediatric Neurosurgeon at Kings vided into two groups: open/aperta and closed/occulta. Closed spinal dysraphism describes College Hospital, London, and specialises in Paediatric Spinal Pathologies, including Spina Bifida. lesions with intact skin that are usually incidentally discovered on radiographic or physical exam. It is characterised by a disruption in the spinous processes and laminae Correspondence to: (mesenchymal structures) without herniation of underlying abnormal or normal neural Ruth-Mary deSouza, structures through the overlying skin. However open dysraphic lesions require emergent Department of Neurosurgery, King’s College Hospital, Denmark Hill, London, SE5 9RS UK. surgical repair to prevent , and are comprised of a broad spectrum of abnormali - ties. Table 1 summarises the key features of some open and closed dysraphic defects. Conflict of interest statement: The spectrum of spinal dysraphism can also be classified according to the point in spinal No author has a conflict of interest to declare. cord embryological development that the abnormality occurs, which will be described in Provenance and peer review: the following section on embryology of spinal dysraphism and in Figure 1. Submitted and internally reviewed. Embryology To cite: In order to understand the mechanism by which spinal dysraphism occurs, it is important deSouza RM, Church P, Elias T, Frim D. ACNR 2014;14(4):28-33. to appreciate the normal embryology of spinal cord development. This is split into three key stages and at each stage abnormalities can arise that give rise to dysraphic lesions. Essentially closed spinal dysraphism is believed to be a problem of secondary (the third stage of spinal development) whilst open dysraphism is a problem earlier on, during primary neurulation.

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Table 1 – Summary of key open and closed dysraphic conditions

Open dysraphic defects Myelomeningocele Abnormal spinal cord exposed in the midline via defect in the posterior vertebral elements, and skin Meningocele Herniation of the meninges via defect in the posterior vertebral elements and fascia. but covered by skin. The cord is not involved. Failure of anterior neuropore to close leads to herniation of a poorly developed brain – not compatible with life Midline defect with no underlying cord. Associated with anencephaly Open dysraphism in the majority of cases is associated with Chiari 2 malformation and that may require CSF diversion Closed dysraphic defects Thickened filum terminale Filum more than 2 mm in diameter and containing abnormal tissue such as fat and fibrous bands Conus Intradural lipoma attached to the distal cord Lipomyelomeningocele Intradural lipoma attached to the distal spinal cord with the lipoma extending out of the spinal canal owing to a focal expansion of CSF space. Can be felt as a subcutaneous lipoma due to bony defect Bony spur dividing the cord into two separate cords in their own dural sleeves (type 1) or one dural sac (Split Cord malformation) and one cord split by a fibrous band (type 2) Terminal myelocystocele Expansion of the distal end of the central canal leading to an intergluteal closed cystic swelling. Dermal sinus tract Epithelialised tract opens on to the skin and which may extend to the intradural space. Can have associated dermoid or epidermoid Neurenteric usually in the intradural extramedullary plane, lined with gastrointestinal or respiratory epithelium (endodermal origin). Can also be cranial Anterior and lateral They are very rare, and are protrusions of one or more layers of the thecal sac through a defect in the meningoceles vertebrae; may be associated with Currarino’s triad.

Figure 1 – flow chart summarising the aberrant processes and time points in spinal cord development that can lead to spinal dysraphism. Stage of spinal cord development Adapted from Thompson et al, 2014 with permission [1]

Disorder of gastrulation Disorder of primary neurulation Disorder of dysjunction Disorder of secondary neurulation

Neurenteric cysts Myelomeningocele Dermal sinus tract Thickened filum Anterior and lateral meningocele Meningocele Lipomyelomeningocele Myelocystocele Diastematomyelia Anencephaly Cord lipoma

Closed defects Open defects Closed defects Closed defects

Embryology of the normal spinal cord dermal folds that fuse at the anterior and poste - Development of the normal spinal cord may rior neuropores as well as additional midline be understood by viewing it as a three stage fusion points to form the brain (rostral end) process: gastrulation, primary neurulation and and spinal cord (caudal end). This newly secondary neurulation. formed primitive central nervous system from Gastrulation refers to the initial formation of the process of ectodermal fusion then under - a trilaminar plate that contains all three of the goes “dysjunction” – physical separation from germ cell layers (, mesoderm and the rest of the ectoderm which then goes on to endoderm) from which all future tissues will become skin. After dysjunction of neural tissue be derived. Ectoderm will give rise to the from the remainder of the ectoderm that is central nervous system and skin, endoderm to destined to be skin, the process of secondary the viscera; and mesoderm to the musculo- neurulation (essentially caudal spinal develop - skeletal system. During gastrulation, the primi - ment) can begin. tive streak of the embryo gives way to develop - During secondary neurulation from days 28- ment of the midline notochord. After gastrula - 48, a separate cell pool of pluripotent stem tion which lasts from days 16 to 18 primary calls located at the very tail of the embryo, neurulation occurs, followed by secondary termed the “caudal cell mass” gives rise to the neurulation. conus, cauda equina as well as parts of the Essentially primary neurulation will result in genitourinary tract and the hindgut (the the formation of the brain and spinal cord viscera being endodermal). The neural struc - whilst secondary neurulation refers to a sepa - tures arising from the caudal cell mass join the rate process which forms the conus medullaris distal spinal cord that was created by the prior and cauda equina. Primary neurulation (days process of primary neurulation. At this point, Image 1 – Photograph of a tail in a child with closed 18-28) involves the development of two ecto - about eight weeks of gestation, spinal tissue dysraphism.

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extends down to the very bottom of the spinal and caregiver costs. 16 marker prompts further investigations or when column. The next stage is for the spinal cord to Known genetic syndromes associated with the patient has an MRI for clinical suspicion of ascend rostrally in order for the conus to NTDs are trisomies 13 and 18 and Currarino tethered cord or for other indications. assume its usual position in the lumbar region. triad 17 and up to 10% of spina bifida cases are Approximately 6% of closed dysraphism is The basis of this process is by the bony verte - associated with chromosomal defects. 18 associated with Chiari 1 malformation. 20 It is bral column that houses the spinal cord therefore important to evaluate children with growing disproportionately faster than the Diagnosis and presentation occult dysraphism for hydrocephalus and neural elements, resulting in the spinal cord Open dysraphism is diagnosed by antenatal related to a Chiari 1. being elevated proximally up the vertebral ultrasound and foetal MRI. The 2008 NICE column. It is between the 8th and 18th week of guidelines on antenatal care recommend that Introduction to Tethered Cord Syndrome gestation, that the growth when routine ultrasound screening is (TCS) exceeds that of the spinal cord resulting in performed to detect spina bifida, it is not Tethered cord is essentially a clinical diag - “caudal ascent” or migration of the cord to necessary to perform alpha-fetoprotein levels. 19 nosis. It refers to patients with dysraphic anom - leave the thin filum terminale. By the 25th The imaging is also useful to identify associ - alies who have manifested clinical features of week of gestation, the growth subsides and, by ated disorders such as hydrocephalus. Occult neurological, urological and orthopaedic two months of age the tip of the conus dysraphism is not usually identified antenatally. pathology secondary to the dysraphism. The medullaris, the most caudal structure of the It is detected either when a midline cutaneous clinical heterogeneity of tethered cord spinal cord, should be found between L1 and syndrome makes the diagnosis a challenge. It L2. A foetal post mortem MRI study showed Image 2A (top) and 2B (bottom): sagittal and axial T2 MRI may present at birth or be asymptomatic only scans of a child with lumbar lipomyelocele. There is that in 94.8% of cases, the conus had ascended posterior sacral agenesis. The spinal cord is low lying with to progress with growth and may present for to L3 by 40 weeks gestation. 4 Ascension stops the conus lying at S1. The large intradural lipomatous the first time in adulthood. 21 Finally, it may 5,6 extension reaches L4 level superiorly, and herniates through at three months. This process can be inhib - the posterior sacral defect. There is dilation of the central present as new or progressive signs in a patient ited, however, in the presence of spinal spinal canal from T9 - L4, which is at its widest calibre at with a known dysraphic state. The physical dysraphism: the finding of the tip of the conus L2/3. The cervical and upper thoracic spinal cord are findings of occult spinal dysraphism do not normal. medullaris below the L1/2 junction can be necessarily correlate with the development of considered pathological. A normally posi - clinical tethered cord syndrome. Tethered tioned conus does not exclude dysraphism. 7 cord syndrome is characterised by the Occult dysraphic pathologies are believed to progressive development of sensorimotor occur from failures of secondary neurological dysfunction, sphincter and closure and failures at the dysjunction stage. sexual dysfunction, and orthopaedic symp - toms. These signs and symptoms often follow a Epidemiology period of growth, i.e. school age children The incidence of dysraphism is approximately between 5 and 15 years of age, and are the 1 per 500-1 per 1000 live births. 8,9 The exact most common presentation. incidence of closed dysraphism is unknown but is significantly higher than that of open Hypotheses for the pathophysiology dysraphism, which is approximately 6 per of TCS 10,000; 10 Open dysraphism is more common in The terminology “tethered cord” refers to the female than in male children. In open fact that the cord or conus is low lying and was dysraphism, women with a history of a thought to have traction placed on it as the previous pregnancy complicated by vertebral column grows whilst the nerve roots dysraphism carry a 3 to 5% risk of recurrent of the cauda equina are still adherent to the spinal dysraphism. 11,12 The prevalence of all lumbosacral area and cannot ascend in types of dysraphism that could be diagnosed parallel to the growing vertebral column. Nerve at birth in the United States decreased almost root stretch is theorised to result in vascular a quarter between 1995-1996 and 2003-2004 compromise with a decrease in blood flow following food fortification with folic acid. 13 and mitochondrial activity and metabolic Both genetic and environmental factors derangement on the background of struc - have been implicated in the aetiology of turally abnormal neural tissue. 22,23,24 Fibrous and spinal dysraphism. A number of genes have fatty bands were identified in a histopatholog - been investigated and no single causative ical and imaging study of dysraphism 25 and gene has been identified, although genetic these may cause mechanical “tethering”. These pathways in folate and 1-carbon metabolism are believed to be important. 14 Environmental factors which have been demonstrated to be associated with high dysraphism risk are folate deficiency, use of some anti-epileptics, poor socio-economic status, maternal age greater than 40 or less than 19. 15 Geographical varia - tions in the prevalence of dysraphism have been reported in first and third world settings, although no firm conclusions regarding ethnicity being a risk factor have been reached. The socioeconomic burden of dysraphism was investigated in a recent meta-analysis of 14 cost of illness studies which showed that key costs were hospital stay, long term compli - cations of dysraphism throughout adulthood

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hypotheses have been debated in the litera - (red or brown) or frank purple vascular tract , stones and changes in conti - ture, but the pathophysiology of the tethered malformations that are most worrisome are nence. Presentation can be either with failure cord syndrome remains unclear. hemangiomas, which can be large and may be to attain continence or new onset inconti - associated with other markers of dysraphism, nence. It may be subtle, with incomplete Cutaneous manifestations of the such as dimples or hairy patches. Raised voiding, urinary frequency, stress incontinence tethered cord syndrome pigmented vascular lesions are usually associ - and nocturnal enuresis. 35 Up to 25% of chil - The main cutaneous stigmata of tethered cord ated with dysraphism. dren with occult dysraphism will have uncoor - are hairy patches, dermal sinuses, heman - Finally, the finding of a tail or pseudo-tail dinated detrusor and sphincter activity, that giomas, subcutaneous , atretic near the coccyx, or a midline raised can lead to permanent renal damage via high meningoceles, abnormal dimples and skin tags. anywhere along the spine, has a strong correla - bladder pressures and recurrent urinary tract Approximately half of clinically suspicious tion with occult spinal dysraphism. A true tail infections (UTIs). 36 Sphincter function should midline spinal cutaneous lesions are associated is extremely rare and composed of fatty tissue, be investigated early to avoid these complica - with dysraphic pathology. 26 Approximately one vasculature, muscle, and nerve fibres. All of tions. 36 Other urological problems associated third of patients with dysraphism show some these lesions are associated with occult spinal with spinal dysraphism include cryp - cutaneous lesion. 27 Skin manifestations are often dysraphism and should prompt further evalua - torchidism, renal agenesis, horseshoe kidney single but less commonly can be multiple. tion. 33 and less commonly cloacal and bladder One lesion of spinal dysraphism is hypertri - Unfortunately,not all occult dysraphic states exstrophy. 37 chosis, or the “hairy patch”. This lesion is are associated with a cutaneous marker, and Bowel dysfunction without bladder involve - described as a midline often V-shaped patch of those without cutaneous markers may present ment is rare in dysraphism. 38 It may present excessive hair in the newborn, not a collection later when other clinical manifestations of with constipation or fecal incontinence. of sparse hair. Hairy patches associated with dysraphism have developed. Rarely, imperforate anus may be present. Low occult dysraphism are usually well defined and high anorectal malformations are associ - and localised as opposed to hirsutism. Hairy Neurological, orthopaedic, bladder and ated with spinal dysraphism but are not patches can be associated with underlying bowel manifestations of TCS usually directly visible without imaging. 39,40 The split cord malformation. 1 Sensorimotor dysfunction can be from a finding of any of these signs and symptoms The finding of a lipoma, or a subcutaneous combination of upper motor neuron (UMN) should prompt further evaluation into the fat pad, is the most common marker that signi - and lower motor neuron (LMN) dysfunction in possibility of spinal dysraphism as the etiology. fies underlying spinal dysraphism. 28 In addition, the case of a conus lesion and pure LMN signs Sometimes, urogenital, hindgut and dysraphic 80-90% of spinal lipomas have an additional for lesions below the conus. This may present spinal pathologies can constitute one of the cutaneous lesion. 29 The lipoma may be limited as lower extremity weakness and changes in genetic syndromes, such as VACTERL (verte - to the dermis, may extend into the intraspinal sensation. Parents may report a delay in bral defects, anal atresia, cardiac defects, space via a vertebral defect, or may be limited walking or changes in gait with the develop - tracheo-esophageal fistula, renal anomalies, to an intraspinal component, which would be a ment of ataxia (worsening with fatigue or exer - and limb abnormalities), OEIS (omphalocele, radiological finding only. A lipomyelomeningo - cise), development of toe walking and regres - exstrophy of the cloaca, imperforate anus, and cele is a lesion which extends from the dermis sion of motor milestones. In addition, there spinal defects) and Currarino’s triad to the intraspinal space, and should be ruled may be contractures, alterations in tone, and (anorectal malformation, a sacral bony defect out prior to surgical resection of a midline muscle atrophy. The child may complain of and a presacral mass). spinal subcutaneous lipoma suspected to be back pain or radicular pain. Examination of Although it is well documented that sexual purely superficial to the fascia. 28 the back may reveal or abnormal dysfunction can be a consequence of tethered Dimples in the sacrococcygeal area often lumbar lordosis in addition to the cutaneous cord and a complication of any treatment for present as a finding on routine baby checks markers described above. it, the psychosexual effect of tethered cord is a and pathological lesions are characterised by Orthopaedic signs are essentially progres - poorly explored area, although from what specific findings. Normal dimples are seen in sive deformities. They affect the lower limbs evidence is available, only half these patients 4% of infants and are characterised by their and/or the spine. Lower limb deformities are are satisfied with their sex life, mainly inhibited location at the tip of the coccyx, which is the result from imbalance between opposing through incontinence and poor self image. 41 palpable through the dimple. The characteris - muscle groups. These include , varus, tics of a pathological dimple include those valgus and equinus deformities, tight Achilles Diagnosing tethered cord syndrome that are midline, located above the natal cleft tendons, clubbing, talipes, toe clawing, The diagnosis of occult spinal dysraphism can and discharging. Dimples that are deeper or hammertoe, and leg length discrepancy. Spinal be a challenging one to make, as the differen - larger than normal also merit investigation deformities can include scoliosis and sacral tial diagnosis is wide. The benefit unique to and should be evaluated as markers of a dysgenesis. These can be combined with GPs is that of familiarity with the medical dermal sinus tract. Dermal sinus tracts are deformity affecting individual vertebrae history of the child and family over time. Work epithelial lined conduits between the skin and (segmental spinal dysgenesis) such as up of TCS includes a thorough physical exam - underlying planes from superficial fascia hemivertebrae, bifid vertebrae and laminar ination and . The physical exam - down as far as the intradural space. Dermal defects. Complications of orthopaedic lesions ination is centred on cutaneous findings along sinuses are primarily of the midline include low bone density, 34 respiratory with neurological, orthopaedic and sphincter lumbosacral spine (90%), although cases exist dysfunction and spasticity. TCS may be demon - features of cord tethering. of thoracic and cervical dermal sinus tracts 30 strated through a sudden deterioration after In addition to physical examination, and tracts off the midline. 31,32 They are believed straightening or correction in scoliosis is concerns regarding this possibility should be to occur from failed separation between cuta - undertaken before untethering. Sometimes, evaluated with radiological studies. A spine x- neous and neural ectoderms – ie a failure of the orthopaedic complaints may be addressed ray can reveal bony abnormalities associated dysjunction. 17 Dermal sinus tracts may be asso - surgically without suspecting the underlying with a spinal dysraphism. However, it will not ciated with dermoids and less commonly with neurological problems. reveal any cord anatomy and causes unneces - epidermoids and rarely teratomas. 17 TCS may also present with urological mani - sary radiation exposure. For this reason, x-rays Undetected, dermal sinuses can lead to festations. These can affect the structure and are obsolete now in the assessment of occult and progressive neurological function of the lower and upper urinary tracts. dysraphism. Ultrasonographic exam before the deficits. All children with recurrent meningitis The most common urological problems are age of three months has been useful to should be assessed for a dermal sinus tract. neurogenic bladder with associated vesicu - examine the level of the conus and identify Midline lumbosacral skin discolouration oureteric reflux, overflow, recurrent urinary obvious dysraphic lesions. In addition, ultra -

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sonography provides real time data and pulsa - is usually performed in a latex free environ - stabilise. On the other hand, some (0-5%) may tions of the cord can be documented. 42 But ment to avoid sensitisation. deteriorate. Deterioration is, as with the initial Magnetic Resonance Imaging is considered to Spinal cord untethering is routinely diagnosis of tethered cord syndrome, a clin - be the “gold standard” in the investigation of performed under intraoperative neurophysio - ical diagnosis 49 and revision surgery focuses spinal dysraphism. Absence of an abnormality logical motor tract and nerve root monitoring on identification and division of adhesions on plain radiograph or with ultrasound evalua - to allow stimulation of neurologically active followed by a tight dural closure or tion in the face of a cutaneous marker or clin - structures and differentiation between adhe - duraplasty. 48,49 Endoscopic division of adhe - ical findings should prompt further evaluation. sions that are functional from those that are sions may be performed by an experienced If evidence of spinal dysraphism is found, a not. 45 Sensory potentials are of limited value. 46 Paediatric Neurosurgeon. 50 The MDT role is whole cranio-spinal MRI should be performed As complete a resection of the dysraphic that of coordinating care as the child grows to identify any associated , anomaly causing TCS as possible should be into adulthood. Children should be evaluated syringomyelia or hydrocephalus. performed to avoid retethering. The complica - for signs of retethering, which has an associ - tion rate is quite low with experienced ated lifetime risk of approximately 10%, higher Options for managing TCS Paediatric Neurosurgeons. In a series of 238 for those in the subset with more complicated Asymptomatic occult dysraphism is typically cord lipoma resections, the incidence of anatomical repairs. managed with regular outpatient follow up neurological and urological complications Tethered cord patients are a heterogenous and active monitoring for orthopaedic, neuro - was 4.2% and that of CSF leaks was 2.5%. 47 In group of individuals in terms of their biopsy - logical and bladder/bowel changes that may complex cases, only partial untethering may chosocial care needs. 51 Patients with associ - suggest development of the tethered cord be possible. 47 ated hydrocephalus and a CSF shunt in situ syndrome. The management of patients with Active monitoring of conservatively are likely to have a greater biopsychosocial occult dysraphism and clinical evidence of managed and post operative patients is best burden than patients without a shunt. 52 This is tethered cord syndrome is controversial and performed by a multidisciplinary team due to any cognitive impairment from cranial there is no level 1 evidence favouring a partic - composed of a Paediatrician, Neurosurgeon, pathology and also from shunt related ular surgical approach or timing of surgery. Urologist, Rehabilitation Physician, complications. Long term musculoskeletal The vast majority of surgeons would operate if Orthopaedic Surgeon and Physiotherapist. consequences of tethered cord include joint the patient develops a new deficit related to Monitoring consists of serial neurologic contractures, skin ulceration, spasticity, tethered cord. Surgery is clearly indicated for exams, imaging if new clinical features muscle wasting, chronic pain and gait distur - those lesions placing the child in immediate develop, orthopaedic follow up, and evalua - bance. These translate into a variable degree danger, such as dermal sinus tracts associated tion of bladder function. Serial urological of physical impairment and functional with meningitis. The risks of untethering exams with urodynamic evaluation provides disability. Management of these requires surgery are low when performed by an expe - an objective tool for the monitoring of input from Physiotherapists and rienced Paediatric Neurosurgeon in a neuro - abnormalities. Occupational Therapists. Close liaison surgical centre accustomed to this type of between therapists, caregivers and educa - surgery. It is for these reasons that patients The role of the multidisciplinary team in tional institutions can be used to maximise with radiological features of dysraphism will the long term care of TCS patients the child’s participation in school activities. almost universally be referred for considera - Issues presented to the care team in the Care needs associated with a neurogenic tion of surgery. The case for prophylactic immediate post-operative period are prima - bladder include training in home catheterisa - surgery is debated in the literature and is not rily those of wound integrity and hygiene. In tion, voiding techniques and regular follow undertaken in most centres on intact patients; children, especially those with low-lying up to monitor renal function. A number of although there is some evidence that surgery lesions, wound infection becomes a signifi - non surgical and surgical strategies exist for early in the course of clinical presentation, cant risk. There is no evidence supporting the neurogenic bladder and bowel including radical resections and surgery in children prophylactic post operative antibiotics. enemas, stomas and bladder suspension. 36 under the age of two years may be associated Another serious risk is that of wound break - Urology specialist nurses and continence with favourable neurological outcome. 43,44 down and/or (CSF) nurses can teach catheterisation, advise on In patients who undergo surgical manage - leakage. Children may be placed on flat bed hygiene and work with patients and their ment, the primary goal of the repair is to rest for 48 hours or more postoperatively to families to minimise the complications and untether the cord and preserve or improve reduce the CSF pressure in the lumbar canal. social stigma associated with continence function. The secondary goal is the repair of The duration for maintaining a flat posi - difficulties. Women with tethered cord need the other associated anomalies available tioning is controversial. A longer period is specialised care during pregnancy. 53 In addi - within the surgical exposure, such as lipomas often necessary after a tenuous dural repair. tion to the physical care requirements of the and sinus tracts that currently or could in Open CSF leak out of the wound should be spina bifida patient, there are significant future contribute to tethering of the cord. This addressed with some urgency for fear of infec - psychological and social aspects affecting procedure may not extend to total resection tion leading to meningitis. The CSF leak may the families of patients. A meta-analysis of 15 of these anomalies if they are not responsible however be contained under the skin in a studies addressing parental psychological for the traction and tethering on the cord. pseudomeningocele. In this scenario, symp - adjustment to spina bifida highlighted aware - In surgery, patients are positioned prone toms of a low-pressure headache may be ness of maternal psychological stress in and a midline incision is made centred over present. External pressure dressings to oblit - particular. 54 The involvement of Pain the area of pathology. The surgical approach erate subcutaneous dead space, CSF diver - Physicians and Psychologists should be tracks the abnormality from the skin, when sion or operative wound revision may need to considered. 55 A family centred rehabilitation present, through potentially abnormal subcu - be performed. model can address the needs of the family taneous tissues, fascia, bifid structures, and Despite surgical repair, a child with occult unit as well as the individual, 56 but independ - into the spinal cord or the filum terminale spinal dysraphism and/or tethered cord ently addressing the psychological care of anomaly. The remainder of the operation syndrome needs to be on close follow up the family, so it does not become entirely focuses on the anatomical untethering of the throughout his or her life. The success rate of focused on the progress of the child. 57 attachments between the spinal cord and untethering is dependent on the cause of teth - Throughout the child’s life, his/her normal surrounding structures. Once the spinal cord ering, with more complex aetiologies making development must be emphasised. This can is untethered, dural closure and closure of the recurrence more likely. 48 Most patients, after truly be a challenge to the family faced with overlying tissues is performed. The procedure surgery, improve or have symptoms that chronic health care issues. Stress should be

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placed on emphasising the normalcy of the Conclusion multidisciplinary team, headed by the child’s child, particularly the normal cognitive devel - The finding of a midline spinal dysraphic primary caregiver. Management options opment (in cases without co-existent cranial defect in a child is often subtle and can be continue to be debated as not all low-lying pathology that causes cognitive impairment), missed. Cutaneous lesions can, however, repre - spinal cords progress to tethered cord the need for normal treatment in relation to sent a marker of an occult spinal dysraphic syndrome. Regardless, the role of the General other children in the household, and the aware - state and therefore suspicious lesions need Practitioner and the Paediatrician in the initial ness of the family to the “vulnerable child” trap. further evaluation. The necessity of evaluation evaluation and communication to families of Despite our inherent desire to protect the child is due to the possibility of a spinal dysraphic these possibilities, and his/her awareness to the from unnecessary difficulties or challenges in state that can lead to the clinical entity of teth - impact of these diagnoses is critical in the opti - life, we may unfortunately contribute further to ered cord syndrome. This diagnosis, its subse - misation of the child’s physical and develop - the handicap by doing so. quent evaluation and management requires a mental health. N

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